Synced here

S+S:

• Unpredictable swelling involving face, oropharynx, extremities, abdomen WITHOUT hives or pruritus
• Laryngeal attacks carry a risk of asphyxiation, making untreated events potentially fatal
• Classically - unexplained, non-pruritic, non-pitting swelling without hives or when allergy-direct treatments fail
• DOES NOT respond to antihistamines, epinephrine, or corticosteroids

Etiology: Autosomal Dominant deficiency of C1-inhibitor function

Triggers:

• direct mechanical friction
• trauma
• viral infection
• contact sports

Investigations:

• Normal C3
• Low C4 (C4 converts to C1)
• C1-INH function

Indications to intubate:

• dysphagia, stridor, voice changes, dyspnea

Management:

• ABCs
• Pain control
• Human C1 esterase concentrate (Canadian Blood Services and Héma-Québec
• Icatibant - SQ bradykinin receptor antagonist
• Prophylaxis: prescribed by HAE specialist
  • SQ C1-INH
  • Lanadelumab
  • Berotralstat

Other Links:

• CMAJ - Hereditary Angioedema
• Canadian Hereditary Angioedema Network